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Epidermolysis Bullosa Simplex Superficialis: A New Variant of Epidermolysis Bullosa Characterized by Subcorneal Skin Cleavage Mimicking Peeling Skin Syndrome

Epidermolysis Bullosa Simplex Superficialis: A New Variant of Epidermolysis Bullosa Characterized... Abstract • We report a new variant of epidermolysis bullosa simplex (EBS), termed EBS superficialis, which is characterized by the development of skin cleavage just beneath the level of stratum corneum. In two of seven patients identified, a second and more focal cleft within the lower third of the epidermis was also detected. Epidermolysis bullosa simplex superficialis appears to be transmitted as an autosomal dominant trait. It differs from other autosomal dominant forms of EBS by the common findings of milia and atrophic scarring, as well as involvement of oral and/or ocular surfaces. Epidermolysis bullosa simplex superficialis is further differentiated from peeling skin syndrome by the presence of blisters and the absence of spontaneous continual exfoliation or peeling. (Arch Dermatol. 1989;125:633-638) References 1. Fine JD. Epidermolysis bullosa: clinical aspects, pathology, and recent advances in research . Int J Dermatol . 1986;25:143-157.Crossref 2. Cooper TW, Bauer EA. Epidermolysis bullosa: a review . Pediatr Dermatol . 1984;1:181-188.Crossref 3. Pearson RW. Clinicopathologic types of epidermolysis bullosa and their nondermatological complications . Arch Dermatol . 1988;124:718-725.Crossref 4. Gedde-Dahl T Jr. Sixteen types of epidermolysis: on the clinical discrimination, therapy, and prenatal diagnosis . Acta Derm Venereol Suppl (Stockh) . 1981;95:74-87. 5. Pearson RW. Histopathologic and ultrastructural findings in certain genodermatoses . Clin Dermatol . 1985;3:143-174.Crossref 6. Levy SB, Goldsmith LA. The peeling skin syndrome . J Am Acad Dermatol . 1982;7:606-613.Crossref 7. Abdel-Hafez K, Safer AM, Selim MM, Rehak A. Familial continual skin peeling . Dermatologica . 1983;166:23-31.Crossref 8. Heid E, Harbit RB, Lazrak B. Desquamation familiale continue . Ann Dermatol Venereol . 1983;110:141-143. 9. Mevorah B, Frenk E, Saurat JH, Siegenthaler G. Peeling skin syndrome: a clinical, ultrastructural and biochemical study . Br J Dermatol . 1987;116:117-125.Crossref 10. Kurban AK, Azar HA. Familial continual skin peeling . Br J Dermatol . 1969;81:191-195.Crossref 11. Silverman AK, Ellis CN, Beals TF, Woo TY. Continual skin peeling syndrome: an electron microscopic study . Arch Dermatol . 1986;122:71-75.Crossref 12. Dicken CH. Peeling skin syndrome . J Am Acad Dermatol . 1985;13:158-160.Crossref 13. Hacham-Zadeh S, Holubar K. Skin peeling syndrome in a Kurdish family . Arch Dermatol . 1985;121:545-546.Crossref 14. Panja SK, Sengupta S. Idiopathic deciduous skin . Int J Dermatol . 1982;21:262-264.Crossref 15. Hintner H, Stingl G, Schuler G, et al. Immunofluorescence mapping of antigenic determinants within the dermal-epidermal junction in mechanobullous diseases . J Invest Dermatol . 1981;76:113-118.Crossref 16. Fine JD, Hintner H, Katz SI. Immunofluorescence studies in epidermolysis bullosa utilizing polyclonal and monoclonal antibodies . In: Beutner EH, Chorzelski TP, eds. Immunopathology of the Skin . 3rd ed. New York, NY: John Wiley & Sons Inc; 1986:399-405. 17. Holbrook KA. Extracutaneous epithelial involvement in inherited epidermolysis bullosa . Arch Dermatol . 1988;124:726-731.Crossref 18. Wright JT, Capps J, Johnson LB, Fine JD. Oral and ultrastructural dental manifestations of epidermolysis bullosa . J Dental Res . 1988;67:249. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Epidermolysis Bullosa Simplex Superficialis: A New Variant of Epidermolysis Bullosa Characterized by Subcorneal Skin Cleavage Mimicking Peeling Skin Syndrome

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Publisher
American Medical Association
Copyright
Copyright © 1989 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1989.01670170047006
Publisher site
See Article on Publisher Site

Abstract

Abstract • We report a new variant of epidermolysis bullosa simplex (EBS), termed EBS superficialis, which is characterized by the development of skin cleavage just beneath the level of stratum corneum. In two of seven patients identified, a second and more focal cleft within the lower third of the epidermis was also detected. Epidermolysis bullosa simplex superficialis appears to be transmitted as an autosomal dominant trait. It differs from other autosomal dominant forms of EBS by the common findings of milia and atrophic scarring, as well as involvement of oral and/or ocular surfaces. Epidermolysis bullosa simplex superficialis is further differentiated from peeling skin syndrome by the presence of blisters and the absence of spontaneous continual exfoliation or peeling. (Arch Dermatol. 1989;125:633-638) References 1. Fine JD. Epidermolysis bullosa: clinical aspects, pathology, and recent advances in research . Int J Dermatol . 1986;25:143-157.Crossref 2. Cooper TW, Bauer EA. Epidermolysis bullosa: a review . Pediatr Dermatol . 1984;1:181-188.Crossref 3. Pearson RW. Clinicopathologic types of epidermolysis bullosa and their nondermatological complications . Arch Dermatol . 1988;124:718-725.Crossref 4. Gedde-Dahl T Jr. Sixteen types of epidermolysis: on the clinical discrimination, therapy, and prenatal diagnosis . Acta Derm Venereol Suppl (Stockh) . 1981;95:74-87. 5. Pearson RW. Histopathologic and ultrastructural findings in certain genodermatoses . Clin Dermatol . 1985;3:143-174.Crossref 6. Levy SB, Goldsmith LA. The peeling skin syndrome . J Am Acad Dermatol . 1982;7:606-613.Crossref 7. Abdel-Hafez K, Safer AM, Selim MM, Rehak A. Familial continual skin peeling . Dermatologica . 1983;166:23-31.Crossref 8. Heid E, Harbit RB, Lazrak B. Desquamation familiale continue . Ann Dermatol Venereol . 1983;110:141-143. 9. Mevorah B, Frenk E, Saurat JH, Siegenthaler G. Peeling skin syndrome: a clinical, ultrastructural and biochemical study . Br J Dermatol . 1987;116:117-125.Crossref 10. Kurban AK, Azar HA. Familial continual skin peeling . Br J Dermatol . 1969;81:191-195.Crossref 11. Silverman AK, Ellis CN, Beals TF, Woo TY. Continual skin peeling syndrome: an electron microscopic study . Arch Dermatol . 1986;122:71-75.Crossref 12. Dicken CH. Peeling skin syndrome . J Am Acad Dermatol . 1985;13:158-160.Crossref 13. Hacham-Zadeh S, Holubar K. Skin peeling syndrome in a Kurdish family . Arch Dermatol . 1985;121:545-546.Crossref 14. Panja SK, Sengupta S. Idiopathic deciduous skin . Int J Dermatol . 1982;21:262-264.Crossref 15. Hintner H, Stingl G, Schuler G, et al. Immunofluorescence mapping of antigenic determinants within the dermal-epidermal junction in mechanobullous diseases . J Invest Dermatol . 1981;76:113-118.Crossref 16. Fine JD, Hintner H, Katz SI. Immunofluorescence studies in epidermolysis bullosa utilizing polyclonal and monoclonal antibodies . In: Beutner EH, Chorzelski TP, eds. Immunopathology of the Skin . 3rd ed. New York, NY: John Wiley & Sons Inc; 1986:399-405. 17. Holbrook KA. Extracutaneous epithelial involvement in inherited epidermolysis bullosa . Arch Dermatol . 1988;124:726-731.Crossref 18. Wright JT, Capps J, Johnson LB, Fine JD. Oral and ultrastructural dental manifestations of epidermolysis bullosa . J Dental Res . 1988;67:249.

Journal

Archives of DermatologyAmerican Medical Association

Published: May 1, 1989

References